Familial Mediterranean Fever developing in a Japanese kidney transplant recipient

Familial Mediterranean Fever (FMF) is an auto-inflammatory disease characterized by periodic febrile episodes and sterile polyserositis and is extremely rare in Asian populations. Here, we report a case of FMF in a 61-year-old Japanese man who received a kidney transplant 31 years ago but had to re-start hemodialysis. Although kidney function had been stable since his initial transplant, serum creatinine levels had been increasing over the 2 years prior to his presentation at our hospital, and a periodic fever developed at the same time. Uremic symptoms were observed, and hemodialysis was re-started, prompting the patient to choose to undergo a second kidney transplantation. We re-checked his medical history and conducted further physical examinations. Given that the patient had previously undergone an operation for olecranon bursitis in which pericardial effusion had been identified, we considered the possibility of FMF and conducted a genetic test, which identified the E202Q heterozygous mutation in the MEFV gene. The patient was therefore diagnosed with variant FMF. To our knowledge, this is the first report of a Japanese kidney transplant recipient being diagnosed as an FMF variant. We describe the relationship of FMF and kidney transplantation in terms of prognosis and important points to note for treatment.